There is nothing more joyful than watching a newborn baby. But sometimes, when that little one starts to cry while sucking milk, and when he suddenly turns blue, every mother and father is scared. Today we are going to talk about a rare but serious condition of the heart that is present at birth, which shows such symptoms. In medicine, we call this Pulmonary Atresia . Don't panic when you hear this name. With the advanced treatments available today, this condition can be successfully dealt with. Let's understand this simply from the beginning.
Simply put, what is Pulmonary Atresia?
To understand this, let's first remember how our heart works. Our heart has four main chambers. The lower chamber on the right (right ventricle) pumps oxygen-poor, impure blood to the lungs to pick up oxygen. The main blood vessel that carries this blood to the lungs is the pulmonary artery.
Imagine, there's a gate-like part between the right ventricle of the heart and this pulmonary artery. We call this the pulmonary valve. This valve opens and closes to send blood to the lungs.
Pulmonary atresia is a condition in which the pulmonary valve does not develop properly while the baby is growing in the womb. Sometimes, this valve can be completely covered by a thick membrane. This means that the main route for blood to flow from the right ventricle to the lungs is completely blocked.
So what happens then? The blood can't get to the lungs to get oxygen. As a result, oxygen-poor, blue blood circulates throughout the body. This is the main reason why the baby's lips, fingertips, and sometimes the entire body look blue. We call this condition cyanosis.
"Alternative pathways" of the heart and variants of this disease
When this main road is blocked, the lungs must somehow get some blood to survive. Fortunately, some "temporary" roads in the heart during pregnancy act as a lifesaver at this time.
- Foramen Ovale: A small hole between the two upper chambers of the heart. This allows oxygen-poor blood to flow from the right side to the left.
- Ductus Arteriosus: A small connection between the main artery that carries blood to the body (aorta) and the artery that carries blood to the lungs (pulmonary artery). Through this, a small amount of blood from the body flows back to the lungs.
It is normal for these temporary passages to close within a few days after birth. However, a baby with Pulmonary Atresia can only survive as long as these passages remain open. Therefore, as soon as the baby is born, doctors will give the baby medication to keep these passages (especially the Ductus Arteriosus) open.
We can see the condition of Pulmonary Atresia in two main ways.
1. Pulmonary Atresia with Intact Ventricular Septum (IVS): Here, the wall between the two lower chambers of the heart (septum) is complete. There are no holes. However, because of this, the right ventricle has no place to pump blood, and it often does not develop properly and is small.
2. Pulmonary Atresia with Ventricular Septal Defect (VSD): Here, in addition to the pulmonary valve being blocked, there is a hole in the wall between the two lower chambers of the heart (Ventricular Septal Defect - VSD). This hole allows blood to flow from the right chamber to the left, so the right chamber may be slightly larger than normal.
Treatment methods are determined based on which of these two types the baby has.
What symptoms can parents look for?
These symptoms usually appear within the first few hours or days after the baby is born. Although the doctors are very attentive to this in the hospital, it is important for you to be aware of this after you get home.
| Symptom | What does this mean? |
|---|---|
| Blue discoloration of the body (Cyanosis) | The lips, tongue, fingertips, and under the nails turn blue or purple. This can increase when the baby cries or drinks milk. This is due to a lack of oxygen in the blood. |
| Rapid or difficult breathing | Even when the baby is just sitting, he or she will breathe very rapidly, seem to be going inward, and gasp. This is an attempt to get the oxygen the body needs. |
| Straining while drinking milk | He sucks some milk, spits it out, stops, and sucks again. Sometimes he falls asleep while drinking. It's hard to continue drinking like a normal baby. |
| Unusual drowsiness and lethargy | The baby is always sleepy. He has no interest in playing or moving his limbs. He feels lifeless. |
| Cold, clammy skin | The baby's hands and feet may feel cold to the touch. Sometimes they may have a sticky, sweaty feel. |
Why did this happen to my baby? What are the risk factors?
This question comes to every parent's mind. "Did I do something wrong?" You may wonder. First of all, the exact cause of this is not yet known. It is not your fault. It is caused by a malfunction in a very complex process that occurs when the baby's heart is formed in the first 8 weeks of pregnancy.
However, several factors have been identified that slightly increase the likelihood, or risk, of this condition occurring.
- Genetic factors: The risk is slightly higher if someone in the family (mother, father) has a congenital heart disease. It can also be associated with certain genetic conditions, such as DiGeorge's syndrome.
- Maternal health during pregnancy: The mother had uncontrolled diabetes during pregnancy.
- Certain medications taken during pregnancy: The use of certain medications can have adverse effects on the fetus (teratogenic). That is why it is advised not to take any medication during pregnancy without the advice of a doctor.
- Smoking: Maternal smoking during or before pregnancy.
- Maternal age: The risk of these types of birth defects may increase slightly as the mother's age increases.
The important thing is that not everyone with these risk factors will develop this condition. Also, even someone without any of these risk factors can develop this condition.
How do doctors find this?
With current technology, this condition can sometimes be diagnosed before the baby is born, that is, during pregnancy .
- During pregnancy: If a routine ultrasound scan shows any abnormalities in your baby's heart, your doctor may refer you to a special scan called a fetal echocardiogram . This allows you to see the structure and function of your baby's heart very clearly.
After the baby is born, if the doctor hears an abnormal heart murmur or sees blue discoloration (cyanosis) while examining the baby, they will perform several tests to confirm this condition.
- Pulse Oximetry: This is a very simple, painless test. A small clip-like device is attached to the baby's big toe and measures the level of oxygen in the blood. If the oxygen level is low, it is a sign of a heart problem.
- Chest X-ray: A chest X-ray can give a rough idea of the size, shape, and blood flow to the lungs.
- Electrocardiogram (ECG/EKG): A test that records the electrical activity of the heart. This can check for any stress on the heart muscle and any changes in the rhythm of the heartbeat.
- Echocardiogram ('Echo' scan): This is the most important and specific test. It is similar to an ultrasound scan. It can clearly see everything in the heart, including the chambers, valves, blood vessels, and how blood flows. Pulmonary Atresia is most accurately diagnosed with this test.
What are the treatments for the baby?
Although this condition cannot be completely cured, surgery and other treatments can significantly improve the blood flow in the baby's heart, allowing them to live a normal life. The treatment plan varies from baby to baby.
Step One: Stabilizing the Baby
As soon as the baby is born, the first goal is to keep the life-saving blood vessel called the Ductus Arteriosus open and prevent it from closing.
- Medication: A medication called alprostadil is given continuously through a vein (IV). This keeps the temporary blood vessel from closing. This creates an additional route for blood to flow to the lungs.
- Other methods: Sometimes, cardiologists use a very thin tube (catheter) that goes through a blood vessel in the leg to the heart and place a small mesh-like piece (stent) inside the Ductus Arteriosus to keep it open.
Step Two: Surgical Procedure
Often, a baby with Pulmonary Atresia will have to undergo a series of surgeries within the first few years of life. These surgeries cannot be done all at once, but are performed in stages as the baby grows.
This series of surgeries is like creating a completely new "pipeline" for blood flow in the heart. All of this is decided by the team of cardiologists and surgeons treating your baby.
The three surgical procedures that are usually performed are:
1. BTT Shunt (First surgery - in the first few weeks): This involves connecting a small artificial tube (shunt) between a branch of a main blood vessel that carries blood to the body and the vessel that carries blood to the lungs. This provides a stable blood supply to the lungs.
2. Glenn Procedure (second surgery - around 4-8 months): When the baby is a little older, the previously placed shunt is removed. Then, the oxygen-poor blood coming from the upper body is connected directly to the pulmonary artery. This greatly reduces the workload on the heart.
3. Fontan Procedure (third and final surgery - around 2-4 years of age): In this, the deoxygenated blood from the lower body is also connected directly to the pulmonary artery. After this surgery, the blood flow is adjusted so that all the deoxygenated blood from the body goes directly to the lungs, instead of going to the right side of the heart.
After these surgeries, the baby will have to stay in the hospital, in the intensive care unit (ICU), for one or two weeks.
How is life after surgery?
After this series of surgeries, the child will be able to go to school and play normally. However, this is a condition that requires lifelong medical supervision.
- Regular medical check-ups: You should go for regular follow-up appointments with a cardiologist. You will need to have tests like an echo scan and ECG at least once a year.
- Complications: Over time, there is a risk of complications such as arrhythmia, liver problems, and heart failure. This is why regular checkups are important.
- Protecting against infections: These children are at increased risk of developing endocarditis, an infection of the lining of the heart. Therefore, you should ask your doctor if you need to take antibiotics to prevent infection before certain medical procedures, such as tooth extractions.
- Activities: You should talk to your doctor and decide what exercises and sports your child can and cannot do.
Words cannot express the pain, fear, and shock you feel when you learn that your child has this condition. But remember, you are not alone. A large team of skilled doctors and nurses are dedicated to providing the best care for your child. Talk to your doctor about any questions or concerns you may have.
Take-Home Message
- Pulmonary atresia is a serious congenital heart disease in which the valve that carries blood to the lungs is blocked.
- The main symptoms are blue discoloration of the baby's body, lips, and fingers (cyanosis), choking while breastfeeding, and difficulty breathing.
- This is a fatal condition if left untreated. However, with the advancement of modern medicine, a series of surgeries can give the child a better life.
- These children require lifelong supervision by a cardiologist and regular medical checkups.
- As parents, never be afraid to ask your doctor questions, clarify doubts, and be well-informed about the treatment plan.
💬 අදහස් (0)
තවමත් කිසිදු අදහසක් පළ කර නොමැත. ඔබේ අදහස පළමු වරට මෙහි එක් කරන්න.
ඔබේ අදහස එක් කරන්න